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The use of Trichomonas vaginalis purine nucleoside phosphorylase to activate fludarabine in the treatment of solid tumors.

Parker WB, Allan PW, Waud WR, Hong J, Gilbert-Ross M, Achyut BR, Joshi D, Behbahani T, Rab R, Ealick SE, Sorscher EJ.

Cancer Chemother Pharmacol. 2020 Jan 8. doi: 10.1007/s00280-019-04018-7. [Epub ahead of print]


Cystic fibrosis precision therapeutics: Emerging considerations.

Joshi D, Ehrhardt A, Hong JS, Sorscher EJ.

Pediatr Pulmonol. 2019 Nov;54 Suppl 3:S13-S17. doi: 10.1002/ppul.24547. Review.


Slowing ribosome velocity restores folding and function of mutant CFTR.

Oliver KE, Rauscher R, Mijnders M, Wang W, Wolpert MJ, Maya J, Sabusap CM, Kesterson RA, Kirk KL, Rab A, Braakman I, Hong JS, Hartman JL 4th, Ignatova Z, Sorscher EJ.

J Clin Invest. 2019 Dec 2;129(12):5236-5253. doi: 10.1172/JCI124282.


VX-770-mediated potentiation of numerous human CFTR disease mutants is influenced by phosphorylation level.

Cui G, Stauffer BB, Imhoff BR, Rab A, Hong JS, Sorscher EJ, McCarty NA.

Sci Rep. 2019 Sep 17;9(1):13460. doi: 10.1038/s41598-019-49921-4.


Making precision medicine personal for cystic fibrosis.

Manfredi C, Tindall JM, Hong JS, Sorscher EJ.

Science. 2019 Jul 19;365(6450):220-221. doi: 10.1126/science.aaw0553. No abstract available.


Assessing cell-specific effects of genetic variations using tRNA microarrays.

Polte C, Wedemeyer D, Oliver KE, Wagner J, Bijvelds MJC, Mahoney J, de Jonge HR, Sorscher EJ, Ignatova Z.

BMC Genomics. 2019 Jul 16;20(Suppl 8):549. doi: 10.1186/s12864-019-5864-1.


Intratumoral generation of 2-fluoroadenine to treat solid malignancies of the head and neck.

Behbahani TE, Rosenthal EL, Parker WB, Sorscher EJ.

Head Neck. 2019 Jun;41(6):1979-1983. doi: 10.1002/hed.25627. Epub 2019 Jan 11. Review.


A Comparison between Two Pathophysiologically Different yet Microbiologically Similar Lung Diseases: Cystic Fibrosis and Chronic Obstructive Pulmonary Disease.

Fenker DE, McDaniel CT, Panmanee W, Panos RJ, Sorscher EJ, Sabusap C, Clancy JP, Hassett DJ.

Int J Respir Pulm Med. 2018;5(2). pii: 098. doi: 10.23937/2378-3516/1410098. Epub 2018 Nov 29.


Non-obstructive vas deferens and epididymis loss in cystic fibrosis rats.

Plyler ZE, Birket SE, Schultz BD, Hong JS, Rowe SM, Petty CF, Crowley MR, Crossman DK, Schoeb TR, Sorscher EJ.

Mech Dev. 2019 Feb;155:15-26. doi: 10.1016/j.mod.2018.10.002. Epub 2018 Nov 2.


Residual function of cystic fibrosis mutants predicts response to small molecule CFTR modulators.

Han ST, Rab A, Pellicore MJ, Davis EF, McCague AF, Evans TA, Joynt AT, Lu Z, Cai Z, Raraigh KS, Hong JS, Sheppard DN, Sorscher EJ, Cutting GR.

JCI Insight. 2018 Jul 26;3(14). pii: 121159. doi: 10.1172/jci.insight.121159. eCollection 2018 Jul 26.


CFTR modulator theratyping: Current status, gaps and future directions.

Clancy JP, Cotton CU, Donaldson SH, Solomon GM, VanDevanter DR, Boyle MP, Gentzsch M, Nick JA, Illek B, Wallenburg JC, Sorscher EJ, Amaral MD, Beekman JM, Naren AP, Bridges RJ, Thomas PJ, Cutting G, Rowe S, Durmowicz AG, Mense M, Boeck KD, Skach W, Penland C, Joseloff E, Bihler H, Mahoney J, Borowitz D, Tuggle KL.

J Cyst Fibros. 2019 Jan;18(1):22-34. doi: 10.1016/j.jcf.2018.05.004. Epub 2018 Jun 20. Review.


Development of an airway mucus defect in the cystic fibrosis rat.

Birket SE, Davis JM, Fernandez CM, Tuggle KL, Oden AM, Chu KK, Tearney GJ, Fanucchi MV, Sorscher EJ, Rowe SM.

JCI Insight. 2018 Jan 11;3(1). pii: 97199. doi: 10.1172/jci.insight.97199. eCollection 2018 Jan 11.


Reduced bone length, growth plate thickness, bone content, and IGF-I as a model for poor growth in the CFTR-deficient rat.

Stalvey MS, Havasi V, Tuggle KL, Wang D, Birket S, Rowe SM, Sorscher EJ.

PLoS One. 2017 Nov 30;12(11):e0188497. doi: 10.1371/journal.pone.0188497. eCollection 2017.


Use of E. coli Purine Nucleoside Phosphorylase in the Treatment of Solid Tumors.

Parker WB, Sorscher EJ.

Curr Pharm Des. 2017 Nov 8. doi: 10.2174/1381612823666171109101851. [Epub ahead of print]


Transformative therapies for rare CFTR missense alleles.

Oliver KE, Han ST, Sorscher EJ, Cutting GR.

Curr Opin Pharmacol. 2017 Jun;34:76-82. doi: 10.1016/j.coph.2017.09.018. Epub 2017 Oct 13. Review.


Increasing the Endoplasmic Reticulum Pool of the F508del Allele of the Cystic Fibrosis Transmembrane Conductance Regulator Leads to Greater Folding Correction by Small Molecule Therapeutics.

Chung WJ, Goeckeler-Fried JL, Havasi V, Chiang A, Rowe SM, Plyler ZE, Hong JS, Mazur M, Piazza GA, Keeton AB, White EL, Rasmussen L, Weissman AM, Denny RA, Brodsky JL, Sorscher EJ.

PLoS One. 2016 Oct 12;11(10):e0163615. doi: 10.1371/journal.pone.0163615. eCollection 2016.


Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseases.

Sabusap CM, Wang W, McNicholas CM, Chung WJ, Fu L, Wen H, Mazur M, Kirk KL, Collawn JF, Hong JS, Sorscher EJ.

JCI Insight. 2016 Sep 8;1(14). pii: e86581.


Trafficking and function of the cystic fibrosis transmembrane conductance regulator: a complex network of posttranslational modifications.

McClure ML, Barnes S, Brodsky JL, Sorscher EJ.

Am J Physiol Lung Cell Mol Physiol. 2016 Oct 1;311(4):L719-L733. doi: 10.1152/ajplung.00431.2015. Epub 2016 Jul 29. Review.


Ribosomal Stalk Protein Silencing Partially Corrects the ΔF508-CFTR Functional Expression Defect.

Veit G, Oliver K, Apaja PM, Perdomo D, Bidaud-Meynard A, Lin ST, Guo J, Icyuz M, Sorscher EJ, Hartman JL IV, Lukacs GL.

PLoS Biol. 2016 May 11;14(5):e1002462. doi: 10.1371/journal.pbio.1002462. eCollection 2016 May. Erratum in: PLoS Biol. 2016 Nov 2;14 (11):e1002574.


Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators.

Wang W, Hong JS, Rab A, Sorscher EJ, Kirk KL.

PLoS One. 2016 Mar 23;11(3):e0152232. doi: 10.1371/journal.pone.0152232. eCollection 2016.


Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy.

Birket SE, Chu KK, Houser GH, Liu L, Fernandez CM, Solomon GM, Lin V, Shastry S, Mazur M, Sloane PA, Hanes J, Grizzle WE, Sorscher EJ, Tearney GJ, Rowe SM.

Am J Physiol Lung Cell Mol Physiol. 2016 May 15;310(10):L928-39. doi: 10.1152/ajplung.00395.2015. Epub 2016 Mar 11.


Cystic fibrosis transmembrane conductance regulator activation by the solvent ethanol: implications for topical drug delivery.

Cho DY, Skinner D, Zhang S, Fortenberry J, Sorscher EJ, Dean NR, Woodworth BA.

Int Forum Allergy Rhinol. 2016 Feb;6(2):178-84. doi: 10.1002/alr.21638. Epub 2015 Dec 3.


From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations.

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL.

Mol Biol Cell. 2016 Feb 1;27(3):424-33. doi: 10.1091/mbc.E14-04-0935.


Channel Gating Regulation by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) First Cytosolic Loop.

Ehrhardt A, Chung WJ, Pyle LC, Wang W, Nowotarski K, Mulvihill CM, Ramjeesingh M, Hong J, Velu SE, Lewis HA, Atwell S, Aller S, Bear CE, Lukacs GL, Kirk KL, Sorscher EJ.

J Biol Chem. 2016 Jan 22;291(4):1854-65. doi: 10.1074/jbc.M115.704809. Epub 2015 Dec 1.


SNP Formation Bias in the Murine Genome Provides Evidence for Parallel Evolution.

Plyler ZE, Hill AE, McAtee CW, Cui X, Moseley LA, Sorscher EJ.

Genome Biol Evol. 2015 Aug 6;7(9):2506-19. doi: 10.1093/gbe/evv150.


Chlorogenic Acid Activates CFTR-Mediated Cl- Secretion in Mice and Humans: Therapeutic Implications for Chronic Rhinosinusitis.

Illing EA, Cho DY, Zhang S, Skinner DF, Dunlap QA, Sorscher EJ, Woodworth BA.

Otolaryngol Head Neck Surg. 2015 Aug;153(2):291-7. doi: 10.1177/0194599815586720. Epub 2015 May 27.


Phase I dose-escalating trial of Escherichia coli purine nucleoside phosphorylase and fludarabine gene therapy for advanced solid tumors.

Rosenthal EL, Chung TK, Parker WB, Allan PW, Clemons L, Lowman D, Hong J, Hunt FR, Richman J, Conry RM, Mannion K, Carroll WR, Nabell L, Sorscher EJ.

Ann Oncol. 2015 Jul;26(7):1481-7. doi: 10.1093/annonc/mdv196. Epub 2015 Apr 21.


Targeted correction and restored function of the CFTR gene in cystic fibrosis induced pluripotent stem cells.

Crane AM, Kramer P, Bui JH, Chung WJ, Li XS, Gonzalez-Garay ML, Hawkins F, Liao W, Mora D, Choi S, Wang J, Sun HC, Paschon DE, Guschin DY, Gregory PD, Kotton DN, Holmes MC, Sorscher EJ, Davis BR.

Stem Cell Reports. 2015 Apr 14;4(4):569-77. doi: 10.1016/j.stemcr.2015.02.005. Epub 2015 Mar 12.


Longevity and plasticity of CFTR provide an argument for noncanonical SNP organization in hominid DNA.

Hill AE, Plyler ZE, Tiwari H, Patki A, Tully JP, McAtee CW, Moseley LA, Sorscher EJ.

PLoS One. 2014 Oct 28;9(10):e109186. doi: 10.1371/journal.pone.0109186. eCollection 2014.


Sinupret activates CFTR and TMEM16A-dependent transepithelial chloride transport and improves indicators of mucociliary clearance.

Zhang S, Skinner D, Hicks SB, Bevensee MO, Sorscher EJ, Lazrak A, Matalon S, McNicholas CM, Woodworth BA.

PLoS One. 2014 Aug 12;9(8):e104090. doi: 10.1371/journal.pone.0104090. eCollection 2014.


A functional anatomic defect of the cystic fibrosis airway.

Birket SE, Chu KK, Liu L, Houser GH, Diephuis BJ, Wilsterman EJ, Dierksen G, Mazur M, Shastry S, Li Y, Watson JD, Smith AT, Schuster BS, Hanes J, Grizzle WE, Sorscher EJ, Tearney GJ, Rowe SM.

Am J Respir Crit Care Med. 2014 Aug 15;190(4):421-32. doi: 10.1164/rccm.201404-0670OC.


An autoregulatory mechanism governing mucociliary transport is sensitive to mucus load.

Liu L, Shastry S, Byan-Parker S, Houser G, K Chu K, Birket SE, Fernandez CM, Gardecki JA, Grizzle WE, Wilsterman EJ, Sorscher EJ, Rowe SM, Tearney GJ.

Am J Respir Cell Mol Biol. 2014 Oct;51(4):485-93. doi: 10.1165/rcmb.2013-0499MA.


Development and maintenance of a biospecimen repository for clinical samples derived from pulmonary patients.

Schwiebert LM, Estell K, Meadows T, Thannickal VJ, Rowe S, Sorscher EJ, Harris WT, Gaggar A, Dransfield M, de Andrade JA.

Clin Transl Sci. 2014 Aug;7(4):336-41. doi: 10.1111/cts.12161. Epub 2014 May 29.


Interference with ubiquitination in CFTR modifies stability of core glycosylated and cell surface pools.

Lee S, Henderson MJ, Schiffhauer E, Despanie J, Henry K, Kang PW, Walker D, McClure ML, Wilson L, Sorscher EJ, Zeitlin PL.

Mol Cell Biol. 2014 Jul;34(14):2554-65.


Porcine nasal epithelial cultures for studies of cystic fibrosis sinusitis.

Dean N, Ranganath NK, Jones B, Zhang S, Skinner D, Rowe SM, Sorscher EJ, Woodworth BA.

Int Forum Allergy Rhinol. 2014 Jul;4(7):565-70. doi: 10.1002/alr.21335. Epub 2014 Apr 14.


The unfolded protein response affects readthrough of premature termination codons.

Oren YS, McClure ML, Rowe SM, Sorscher EJ, Bester AC, Manor M, Kerem E, Rivlin J, Zahdeh F, Mann M, Geiger T, Kerem B.

EMBO Mol Med. 2014 May;6(5):685-701. doi: 10.1002/emmm.201303347.


Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.

Tuggle KL, Birket SE, Cui X, Hong J, Warren J, Reid L, Chambers A, Ji D, Gamber K, Chu KK, Tearney G, Tang LP, Fortenberry JA, Du M, Cadillac JM, Bedwell DM, Rowe SM, Sorscher EJ, Fanucchi MV.

PLoS One. 2014 Mar 7;9(3):e91253. doi: 10.1371/journal.pone.0091253. eCollection 2014.


S-palmitoylation regulates biogenesis of core glycosylated wild-type and F508del CFTR in a post-ER compartment.

McClure ML, Wen H, Fortenberry J, Hong JS, Sorscher EJ.

Biochem J. 2014 Apr 15;459(2):417-25. doi: 10.1042/BJ20131037.


Marked repression of CFTR mRNA in the transgenic Cftr(tm1kth) mouse model.

Zhang S, Ranganath NK, Skinner D, Bedwell DM, Buckley-Lanier JA, Sorscher EJ, Woodworth BA.

J Cyst Fibros. 2014 May;13(3):351-2. doi: 10.1016/j.jcf.2013.11.009. Epub 2013 Dec 27. No abstract available.


Resveratrol enhances airway surface liquid depth in sinonasal epithelium by increasing cystic fibrosis transmembrane conductance regulator open probability.

Zhang S, Blount AC, McNicholas CM, Skinner DF, Chestnut M, Kappes JC, Sorscher EJ, Woodworth BA.

PLoS One. 2013 Nov 25;8(11):e81589. doi: 10.1371/journal.pone.0081589. eCollection 2013. Erratum in: PLoS One. 2014;9(1). doi:10.1371/annotation/d852ff1d-2824-4f4d-80f2-9be7a42a5f25.


Myofibroblast differentiation and enhanced TGF-B signaling in cystic fibrosis lung disease.

Harris WT, Kelly DR, Zhou Y, Wang D, MacEwen M, Hagood JS, Clancy JP, Ambalavanan N, Sorscher EJ.

PLoS One. 2013 Aug 12;8(8):e70196. doi: 10.1371/journal.pone.0070196. eCollection 2013. Erratum in: PLoS One. 2013;8(8). doi:10.1371/annotation/d0132de3-56ca-4258-9119-bdab0ceb2cff. Macewen, Mark [corrected to MacEwen, Mark].


Comparison of cystic fibrosis transmembrane conductance regulator (CFTR) and ciliary beat frequency activation by the CFTR Modulators Genistein, VRT-532, and UCCF-152 in primary sinonasal epithelial cultures.

Conger BT, Zhang S, Skinner D, Hicks SB, Sorscher EJ, Rowe SM, Woodworth BA.

JAMA Otolaryngol Head Neck Surg. 2013 Aug 1;139(8):822-7. doi: 10.1001/jamaoto.2013.3917. Erratum in: JAMA Otolaryngol Head Neck Surg. 2013 Oct;139(10):1.


The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction.

Lazrak A, Fu L, Bali V, Bartoszewski R, Rab A, Havasi V, Keiles S, Kappes J, Kumar R, Lefkowitz E, Sorscher EJ, Matalon S, Collawn JF, Bebok Z.

FASEB J. 2013 Nov;27(11):4630-45. doi: 10.1096/fj.13-227330. Epub 2013 Aug 1.


Heterozygosity for the F508del mutation in the cystic fibrosis transmembrane conductance regulator anion channel attenuates influenza severity.

Aeffner F, Abdulrahman B, Hickman-Davis JM, Janssen PM, Amer A, Bedwell DM, Sorscher EJ, Davis IC.

J Infect Dis. 2013 Sep 1;208(5):780-9. doi: 10.1093/infdis/jit251. Epub 2013 Jun 7.


Massive microRNA sequence conservation and prevalence in human and chimpanzee introns.

Hill AE, Sorscher EJ.

Front Biosci (Elite Ed). 2013 Jun 1;5:814-22.


Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography.

Liu L, Chu KK, Houser GH, Diephuis BJ, Li Y, Wilsterman EJ, Shastry S, Dierksen G, Birket SE, Mazur M, Byan-Parker S, Grizzle WE, Sorscher EJ, Rowe SM, Tearney GJ.

PLoS One. 2013;8(1):e54473. doi: 10.1371/journal.pone.0054473. Epub 2013 Jan 23.


A yeast phenomic model for the gene interaction network modulating CFTR-ΔF508 protein biogenesis.

Louie RJ, Guo J, Rodgers JW, White R, Shah N, Pagant S, Kim P, Livstone M, Dolinski K, McKinney BA, Hong J, Sorscher EJ, Bryan J, Miller EA, Hartman JL 4th.

Genome Med. 2012 Dec 27;4(12):103. doi: 10.1186/gm404.


A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease.

Sloane PA, Shastry S, Wilhelm A, Courville C, Tang LP, Backer K, Levin E, Raju SV, Li Y, Mazur M, Byan-Parker S, Grizzle W, Sorscher EJ, Dransfield MT, Rowe SM.

PLoS One. 2012;7(6):e39809. doi: 10.1371/journal.pone.0039809. Epub 2012 Jun 29.


In vivo antitumor activity of intratumoral fludarabine phosphate in refractory tumors expressing E. coli purine nucleoside phosphorylase.

Sorscher EJ, Hong JS, Allan PW, Waud WR, Parker WB.

Cancer Chemother Pharmacol. 2012 Aug;70(2):321-9. doi: 10.1007/s00280-012-1908-9. Epub 2012 Jul 4.

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